Trigeminal trophic syndrome: A rare cause of chronic facial pain and skin ulcers

A 25-year-old female presented with chronic right-sided facial pain and ulcers that had gradually developed over four years. Symptoms began after the last of four microvascular decompressions and partial trigeminal nerve section for trigeminal neuralgia. Investigations including biopsy for neoplastic, infectious, and inflammatory conditions were unrevealing. She was diagnosed with trigeminal trophic syndrome (TTS), a consequence of trigeminal nerve injury. This condition was originally described as “trigeminal neuropathy with nasal ulceration.” TTS consists of pain, anesthesia and ulcers in the trigeminal nerve distribution (1–4) (see Figure 1). Although the pathogenesis of TTS remains unknown, a large series of 99 cases has suggested that self-inflicted trauma of the paresthetic skin could be responsible for the lesions and therefore the habit should be abandoned (3). Pain is often refractory to multiple opiate and neuropathic pain medications (1–4).

Figure 1.

Anterior (a) and inferior (b) view of patient’s face, showing scattered ulcers in the right V1–3 distributions. Note loss of lateral nasal ala.